Primary Pachydermoperiostosis and report of two cases in a family

Abstract

Pachydermoperiostosis (PDP) is a rare hereditary disease characterized by folded coarse skin, hyperostosis, clubbing of fingers and abnormalities in other organs such as gastrointestinal tract. Because of safety parents guides us to familial incidence and penetration of gene in 50% of cases. Diagnosis is based on clinical and radiological findings. Males are more susceptible than females to this disease. This is the report of primary PDP in two brothers whose parents were net affected. Therapy in this syndrome is symptomatic, and using of low dose colchicine was not effective in this case.